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Diagnosis and Treatment of AIDS-Related Primary Central Nervous Lymphoma

Dai Watanabe, Yusuke Koizumi, Keishiro Yajima, Tomoko Uehira, and Takuma Shirasaka

Primary central nervous system lymphoma (PCNSL) is an intracranial tumorous lesion that develops in acquired immunodeficiency syndrome (AIDS) patients. PCNSL is very rare, but its prevalence has increased with the expansion of human immunodeficiency virus (HIV) infections. It is diagnosed by imaging or biopsy. Serum and cerebrospinal fluid (CSF) have been analyzed for the presence of diagnostic biomarkers for PCNSL. The presence of Epstein-Barr virus in the CSF is a widely used biomarker, but it has a low positive predictive value. B-cell activation-related cytokines, tumor-specific DNA methylation, and microRNAs have been reported as new candidate biomarkers. Previously, the prognosis of AIDS-related PCNSL was very poor. However, highly active antiretroviral therapy emerged in the late 1990s, which continuously inhibits viral replication and facilitates the recovery of immunity in HIV-infected persons, improving the prognosis of PCNSL patients in combination with whole-brain irradiation. In this review, we focus on the diagnosis of and biomarkers for AIDS-related PCNSL and advancements in its treatment.